Dandy-Walker malformation

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

Large head circumference. A brain CT was performed (not shown), and VP shunt was inserted.

Patient Data

Age: 4 years
Gender: Female
mri
This study is a stack
Sagittal
T1
This study is a stack
Axial
FLAIR
This study is a stack
Axial
T2
This study is a stack
Coronal
T2
This study is a stack
Sagittal
Fiesta
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Info

MRI sequences demonstrate

  • enlarged posterior fossa with a  trocular lying above the level of the lambdoid  (torcular-lambdoid inversion) due to an abnormally high tentorium
  • hypoplasia of the vermis with a small vermian remnant displaced and rotated superiorly
  • cystic dilatation of the 4th ventricle extending posteriorly forming a cystic space with anterolateral displacement of the cerebellar hemispheres.
  • the sagittal Fiesta sequence shows narrowing of the aqueduct of Sylvius with dilated 3rd and lateral ventricles.

The tip of the right VP shunt is well-visualized in projection of the right lateral ventricle.

Case Discussion

Case showing the classic features of a Dandy-Walker malformation with aqueduct stenosis and prominent obstructive hydrocephalus

 

Additional contributor: H. Khouachi, radiographer CIM Aurès Batna Algeria

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